Immunotherapy and radioimmunotherapy for desmoplastic small round cell tumor Review
| Authors: | Espinosa-Cotton, M.; Cheung, N. K. V. |
| Review Title: | Immunotherapy and radioimmunotherapy for desmoplastic small round cell tumor |
| Abstract: | Desmoplastic small round cell tumor (DRSCT) is a highly aggressive primitive sarcoma that primarily affects adolescent and young adult males. The 5-year survival rate is 15-30% and few curative treatment options exist. Although there is no standard treatment for DSRCT, patients are most often treated with a combination of aggressive chemotherapy, radiation, and surgery. Targeted therapy inhibitors of PDGFA and IGF-1R, which are almost uniformly overexpressed in DSRCT, have largely failed in clinical trials. As in cancer in general, interest in immunotherapy to treat DSRCT has increased in recent years. To that end, several types of immunotherapy are now being tested clinically, including monoclonal antibodies, radionuclide-conjugated antibodies, chimeric antigen receptor T cells, checkpoint inhibitors, and bispecific antibodies (BsAbs). These types of therapies may be particularly useful in DSRCT, which is frequently characterized by widespread intraperitoneal implants, which are difficult to completely remove surgically and are the frequent cause of relapse. Successful treatment with immunotherapy or radioimmunotherapy following debulking surgery could eradiate these micrometasteses and prevent relapse. Although there has been limited success to date for immunotherapy in pediatric solid tumors, the significant improvements in survival seen in the treatment of other pediatric solid tumors, such as metastatic neuroblastoma and its CNS spread, suggest a potential of immunotherapy and specifically compartmental immunotherapy in DSRCT. Copyright © 2021 Espinosa-Cotton and Cheung. |
| Keywords: | gene mutation; overall survival; review; sorafenib; bevacizumab; doxorubicin; paclitaxel; temozolomide; dna damage; gene overexpression; interleukin 2; ipilimumab; apoptosis; transforming growth factor beta; etoposide; epidermal growth factor receptor; epidermal growth factor receptor 2; cyclophosphamide; vincristine; cytotoxicity; ifosfamide; cixutumumab; irinotecan; somatomedin c receptor; temsirolimus; immune response; immunotherapy; cancer vaccine; allogeneic hematopoietic stem cell transplantation; natural killer cell; androgen receptor; targeted therapy; antibodies; cytokine release; immunosuppressive treatment; radioimmunotherapy; connective tissue growth factor; desmoplastic small round cell tumor; cholinergic receptor; tumor microenvironment; molecularly targeted therapy; epithelial mesenchymal transition; platelet derived growth factor a; akt signaling; immune checkpoint inhibitor; antibody therapy; interleukin 15 receptor; nivolumab; ramucirumab; human; car t cell; vinorelbine tartrate; dsrct = desmoplastic small round cell tumor; clr 131; enoblituzumab; prexasertib; seclidemstat |
| Journal Title: | Frontiers in Oncology |
| Volume: | 11 |
| ISSN: | 2234-943X |
| Publisher: | Frontiers Media S.A. |
| Date Published: | 2021-11-01 |
| Start Page: | 772862 |
| Language: | English |
| DOI: | 10.3389/fonc.2021.772862 |
| PROVIDER: | scopus |
| PMCID: | PMC8641660 |
| PUBMED: | 34869013 |
| DOI/URL: | |
| Notes: | Review -- Export Date: 3 January 2022 -- Source: Scopus |
